BACKGROUND AND METHODS: Osteonecrosis of the femoral head is an important complication of sickle cell disease. We studied 2590 patients who were over 5 years of age at entry and followed them for an average of 5.6 years. Patients were examined twice a year, and radiographs of the hips were ...

Among 761 consecutive patients with chronic myeloproliferative disorders (CMD), it was found that 18 (nine men and nine women) did not fulfill at presentation the established diagnostic criteria for the typical forms. In seven patients, the diagnosis of CMD was made on the basis of an intense and persistent thrombocytosis ...

Hematological disorders are commonly complicated by anemia, and the symptoms of red cell deficiency adversely affect the quality of life. Erythropoietin is a glycoprotein which controls red blood cell production. Recombinant human erythropoietin, 50 U/kg/day, was given subcutaneously to 16 patients with myelodysplastic syndrome and anemia. All but one patient ...

12 patients with myelodysplastic syndromes were treated with recombinant human erythropoietin (r-epo). 5 patients had stable anemia, 78-92 g/l, and 7 were transfusion-dependent. In 11 patients, r-epo was given intravenously three times a week, with dose escalation after 4 and 8 wk if hemoglobin did not increase more than 15 ...

We reviewed the prevalence of avascular necrosis (AVN) in a series of patients with sickle cell disease, using radiography and magnetic resonance imaging. We found AVN of at least one hip in 11 of 27 patients (41%). This is a significantly greater prevalence than reported. MRI was not as helpful ...

The neurological complications of sickle-cell disease include cerebral intracerebral hemorrhage; subarachnoid hemorrhage (SAH) has been infrequently reported. Among 325 patients with sickle-cell disease followed at the University of Illinois between 1975 and 1989, 11 cases of SAH were identified. Aneurysms were found in 10 of these patients, three of whom ...

Fanconi's anemia (FA) is an autosomal recessive condition in which greater than 90% of the homozygotes develop aplastic anemia. To determine the relation between erythroid progenitors and clinical status, blood and marrow mononuclear cells were cultured in methyl cellulose with erythropoietin, plus other hematopoietic growth factors, and growth in normal ...

A variety of drugs have been tried, with little or no benefit, to prevent and treat painful crises in patients with sickle cell anaemia. The new drug pentoxifylline, which has the ability to alter red cell flexibility, was tried in nine patients with painful vaso-occlusive crises. Another nine matched patients ...

The effect of recombinant human erythropoietin (rHuEPO) on the immune system of hemodialysis patients has been studied by evaluating their response to hepatitis B (HB) vaccination. Fifty hemodialysis patients were given four doses of 20 micrograms recombinant DNA hepatitis B vaccine (SKF) at an interval of 0, 1, 2 and ...

High levels of HbF were found in patients with myelodysplastic syndrome (MDS), as well as a possible switching of the ratio of the gamma chains from the adult to the newborn type in 25% of our patients. These abnormalities in general were not present in the parents. The possibility of ...

Significant numbers of patients receiving azidothymidine (AZT) develop anaemia requiring an adjustment of AZT dosage. Fear of red blood cell (RBC) alloimmunization may act as a deterrent to exposing a patient to long-term transfusion therapy which is the alternative to AZT dosage reduction or discontinuation. This retrospective study was done ...

The incidence of posttransfusion hepatitis (PTH) was determined prospectively at our institution. An active surveillance program of transfused surgical patients was set up; alanine aminotransferase (ALT) levels were determined before transfusion and at monthly intervals for 6 months after transfusion. Patients with confirmed ALT values greater than 2.5 times the ...

The myelodysplastic syndromes are composed of a group of clonal hematologic neoplasms, the course of which is complicated by ineffective hematopoiesis or leukemic transformation (or both). Therapeutic results may have been misleading in the past. Most patients have been managed with supportive measures, such as transfusions of erythrocytes, administration of ...

Five patients with sickle cell haemoglobinopathies underwent open heart surgery. At the start of the cardiopulmonary bypass the patient's circulating blood volume was separately drained into a bag, spun down and retransfused into the patient at the end of the operation. Moderate hypothermia, aortic cross clamping, topical hypothermia and cold ...

The frequency and severity of the painful sickle cell crisis vary greatly among affected patients. Aside from a high level of Hb F(greater than 20%) there is no established parameter which may modulate the clinical severity of the disease. In this paper we describe two groups of adult patients with ...

To determine the prevalence and characteristics of priapism associated with sickle cell hemoglobinopathic conditions we interviewed and examined 52 men with sickle cell anemia, 10 with sickle C disease, 3 with sickle B(+)-thalassemia and 19 with sickle cell trait. Of the men 20 with sickle cell anemia (38%) and 1 ...

The transfusion of red cells (RBCs) was analyzed over a 4-year period (1984-1987), during which 9596 cesarean deliveries were performed. A total of 336 patients were identified as receiving RBC transfusions during or after cesarean delivery; 747 units of RBCs were administered. The overall incidence of transfusion in this patient ...

Serum immunoreactive erythropoietin (EPO) was measured sequentially in 8 patients with thalassaemia major. The EPO levels were distinctly increased before transfusion; they did not significantly change just after transfusion, but subsequently decreased. Pretransfusion EPO levels were similar to those observed in patients with non renal non inflammatory anaemias of the ...

In a study involving 97 patients with hemoglobin SC sickle cell disease (Hb SC) and 87 subjects with hemoglobin SS sickle cell disease (Hb SS) on the island of Curaçao, we found a higher incidence of peripheral retinal vascular closure and proliferative retinopathy in the former as compared with the ...

We examined about half the estimated number (based on gene frequency) of sickle cell patients on the island of Curaçao. This study may represent one of the most comprehensive and a selective cross-sectional surveys available. Studying 81 Hb SS and 97 Hb SC patients, we were able to confirm the ...

NK activity in relation to proliferation of cancer cells was clinically evaluated in the postoperative course in patients with carcinomas of the stomach and colon as compared with that in patients with benign diseases. 1) Preoperative NK activities in patients with advanced cancers were inhibited as compared with those in ...

We evaluated the safety and efficacy of Fluosol DA-20% (FDA) as a blood substitute in the treatment of severe anemia. Thirty-six patients received either FDA (n = 21) or crystalloid/hydroxyethyl starch (CHS) (n = 15) as part of a randomized, controlled trial. Ten patients received FDA as part of a ...

Massive transfusion may cause abnormalities of electrolytes, clotting factors, pH, and temperature and may occur in a scenario of refractory coagulopathy and irreversible shock. Identification of correctable variables to improve survival is complicated by the interplay of this pathophysiology. Temperature may be an under-appreciated problem in the genesis of coagulopathy. ...

We retrospectively reviewed clinical and echocardiographic data on 10 newborns with erythroblastosis fetalis who were admitted to our nurseries between 1984 and 1988 and who required a double-volume exchange transfusion and neonatal intensive care. Echocardiograms were performed in the first 48 hours of life. In 5 patients, disproportionate septal hypertrophy ...

Eight patients with myelodysplastic syndromes (MDS) and four patients with aplastic anemia (AA) were treated with recombinant erythropoietin (rEpo) to investigate its effect on the anemia of these patients. rEpo was administered by i.v. injection three times a week for at least four weeks. The doses were 3,000, 6,000, or ...

Four cases of spontaneous splenic rupture after infectious mononucleosis (IM) have been treated at this institution since 1978. The condition is rare, occurring in 0.1-0.5 per cent of patients with proven infectious mononucleosis. Splenectomy is considered the treatment of choice for these patients. However, because recent trends in the management ...

The potential danger of low oxygen tension to patients with sickle cell disease is widely known. However, less well known is the phenomenon of patients with sickle cell disease presenting with toothache in the absence of any dental pathology. This study investigated the experience of orofacial pain in three matched ...

Patients with sickle cell disease are predisposed to infection caused by Streptococcus pneumoniae. However, there has been only one published case of bacteremic pneumococcal meningitis in an adult with sickle cell anemia. We report here the cases of six adults with sickle cell disease, pneumococcal sepsis, and meningitis. Five patients ...

A retrospective study was performed to estimate the frequency of alloimmunization against red cell (RBC) antigens in a multiply transfused group. Patients (n = 186) were studied who had received at least six blood transfusions during a period of at least 3 months. Some 6944 units of blood were transfused. ...

Hair samples of some young sickle cell anemia (SCA) and control patients in Nigeria were analyzed for 12 elements, viz, Se, Hg, Cr, Fe, Zn, Co, Cu, Br, As, Sb, Na, and Sc, using Instrumental Neutron Activation Analysis (INAA). With the exception of Cu, which was found to be significantly ...

Bone marrow was harvested for the purpose of autologous bone marrow transplantation (ABMT) in 21 patients previously treated with chemotherapy and in complete remission from acute leukemia or non-Hodgkin's lymphoma. The volume required to obtain 2 x 10(8) nucleated cells per kg was less than 15 mL per kg in ...

Because fetal hemoglobin contains gammaglobin chains instead of beta chains, it is not affected by the genetic defect that causes sickle cell disease. Increased levels of fetal hemoglobin decrease the tendency toward intracellular polymerization of sickle hemoglobin that characterizes this disease. Hydroxyurea is one of several cytostatic agents that have ...

Granulocyte transfusions are associated with a number of side effects including febrile transfusion reactions and occasionally pulmonary infiltrates. There is evidence that the presence of preformed antibodies may be a cause of these complications. In this study, allogeneic 111Indium-labeled granulocytes were used to evaluate the pulmonary retention of radioactivity in ...

The effect of hydroxyurea on foetal haemoglobin (HbF) levels was evaluated in 36 patients of myeloproliferative and myelodysplastic disorders. In 17 (47.2%) patients, HbF levels increased from 1.40 +/- 1.17 to 3.03 +/- 1.97 per cent after 4 wk therapy with hydroxyurea. In the responders this increase was highly significant ...

The clinical and haematological features of homozygous sickle cell (SS) disease were compared in 30 Greek and 310 Jamacian patients. Deletional alpha-thalassaemia, which modifies SS disease, is rare among Greek patients, so only Jamacian patients with four alpha-globin genes were included in the control group. Greek patients had higher total ...

Excess red blood cells (RBC) in patients with polycythemia vera (PV) are usually removed by repeated phlebotomy. In order to improve the efficacy of this treatment, we used isovolemic large-volume erythrocytapheresis (EA) by a cell separator. A retrospective analysis of our experience with 69 PV patients (206 EA procedures) is ...

The patient population for elective orthognathic surgery usually comprises young, healthy patients, in whom homologous blood transfusions should be avoided. Homologous transfusion poses substantial, potentially preventable risks to this patient population. In addition to autologous predonation, acute intentional hemodilution is an alternative that potentially avoids the use of homologous blood. ...

Hemoglobinopathies are a major public health problem in Saudi Arabia. We studied the effect of splenectomy in 16 Saudi Arabian children with compound hemoglobinopathies. Seven patients no longer require regular blood transfusions, and transfusion requirements were decreased by 30 to 60% in the other eight patients. Three patients whose heights ...

We present the results of tests carried out to detect alloimmunization against red cells in 1,200 patients (607 males and 593 females), transfused and followed up during the period 1981-1987 in our hospital. Of these patients, 1,135 were thalassemic and 65 had sickle cell/beta-thalassemia. In 162 patients who received blood ...

The frequency of antinuclear antibodies (ANA) and other antinuclear factors was prospectively evaluated in patients with sickle cell disease (SCD). Ten of 44 patients studied (22.7%) had positive ANA determinations at titers greater than or equal to 1:40 compared to 3 of 46 healthy controls (6.5%; p less than 0.03). ...

Haptoglobin types were determined in 626 individuals living in the State of São Paulo (Brazil). Of these, 484 had Hb AA, 31 major beta thalassemia, 43 minor beta thalassemia, 14 Hb SS, and 54 Hb AS. Frequency distribution of the three most common types observed among patients with type beta ...

The kidney is involved in virtually all individuals who inherit the sickle cell form of hemoglobin. Though asymptomatic and relatively common, proteinuria in patients with sickle cell anemia (SS) over 40 years old is associated with reduced creatinine clearance. The subclinical increase in urinary albumin is termed microalbuminuria and is ...

We previously reported that myleran, a cell cycle nonspecific drug, can stimulate gamma-globin gene expression in anemic adult rhesus monkeys. This finding prompted us to treat two patients with severe beta-thalassemia with myleran. Both patients received an initial course of therapy, constantly of myleran at a dosage of 0.2 mg/kg/d ...